Thoracoscopic Management of Recurrent Pneumothorax in an Adult Patient with Cri du Chat Syndrome and Severe Scoliosis: A Case Report.

INTRODUCTION

Cri du chat syndrome (CdCS) is a rare genetic disorder that can be associated with scoliosis and marked thoracic deformity. Although scoliosis has been reported to be associated with an increased incidence of pneumothorax, reports of pneumothorax surgery in patients with severe scoliosis associated with CdCS are extremely rare. Herein, we report a case of recurrent pneumothorax in an adult patient with CdCS associated with severe scoliosis who was successfully treated by thoracoscopic surgery.

CASE PRESENTATION: A 41-year-old man with CdCS presented with severe scoliosis, intellectual disability, and dental abnormalities. He was transported to our emergency department with severe left pneumothorax associated with complete left lung collapse and marked hypoxemia. CT after chest drainage revealed a bulla located in the deep thoracic cavity near the mediastinum. Because severe thoracic deformity raised concerns regarding both airway management and thoracoscopic manipulation, the patient was initially managed conservatively. However, severe left pneumothorax repeatedly recurred, and surgical intervention was ultimately indicated. Thoracoscopic surgery was performed under 1-lung ventilation using a bronchial blocker. Intrathoracic manipulation was technically challenging because of the marked thoracic deformity associated with severe scoliosis; however, safe operative exposure was achieved using same-axis thoracoscopic manipulation with the thoracoscope and instruments through the same port. A broad-based causative bulla located near the S6-S10 boundary adjacent to the descending thoracic aorta was ligated using ENDOLOOP, followed by intrathoracic administration of 20 mL of 50% glucose solution. The postoperative course was uneventful with no recurrence during 7 years of follow-up.

CONCLUSIONS

In patients with severe scoliosis associated with CdCS, thoracic deformity may complicate both airway management and thoracoscopic manipulation. However, thoracoscopic surgery may be feasible with appropriate anesthetic planning and adaptation of the operative strategy. In particular, same-axis thoracoscopic manipulation using the thoracoscope and instruments through the same port may be useful for maintaining safe visualization and instrument handling within a narrow thoracic cavity.